La Revue de médecine interne
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Myelodysplastic syndromes (MDS) are clonal hematopoietic malignancies which are also characterised by immune dysregulation. The impaired immune response is mainly due to T lymphocytes (CD8 and T regulatory cells) with increased cell apoptosis. MDS could be associated in some cases with various clinical dysimmune features; however, only MDS with trisomy 8 is correlated with particular clinical phenotype. ⋯ The dysimmune manifestations, and among them the Behçet's-like disease, do not impact the overall survival or the risk of progression to acute myeloid leukemia. Immunosuppressive and immunomodulatory therapies, and among them TNF-α inhibitors, are usually ineffective to control the dysimmune manifestations. Targeting the underlying clonal disease with specific therapies, such as azacitidine, seems to be the best strategy to control these disorders, even in MDS patients with low-risk disease.
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The medical treatment of preeclampsia is well structured in its acute phase but the required follow-up with patients in post-partum is discussed. However, preeclampsia is associated with an increased risk of cardiovascular morbi-mortality in the long term. In order to optimize the post-partum treatment, a care program has been developed for these patients in the city of Nantes, France. This includes a check-up of the cardiovascular risks at a day hospital. Our study presents the first results of this program. ⋯ The study confirmed that this care program enables an earlier detection and therefore treatment of the cardiovascular risk factors of these young women.
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Immune Thrombotic Thrombocytopenic Purpura (iTTP) is a rare but severe disease with a mortality rate of almost 100 % in the absence of adequate treatment. iTTP is caused by a severe deficiency in ADAMTS13 activity due to the production of inhibitory antibodies. Age has been shown to be a major prognostic factor. iTTP patients in the elderly (60yo and over) have more frequent organ involvement, especially heart and kidney failures compared with younger patients. They also have non-specific neurologic symptoms leading to a delayed diagnosis. ⋯ Interestingly, the mechanisms involved in endothelial activation may share common features with those involved in vascular aging, potentially leading to endothelial dysfunction. It could thus be interesting to better investigate the causes of mid- and long-term mortality among older iTTP patients to confirm whether inflammation and endothelial activation really impact vascular aging and long-term mortality in those patients, in addition to their presumed role at iTTP acute phase. If so, further insights into the mechanisms involved could lead to new therapeutic targets.
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Erysipelas is defined by a sudden onset (with fever) preceding the appearance of a painful, infiltrated, erythematous plaque, accompanied by regional lymphadenopathy. It is usually localized on the lower limbs, but it can occur on the face. It is due to β-hemolytic streptococcus A and more rarely to staphylococcus aureus. ⋯ In uncomplicated erysipelas, amoxicillin is the gold standard; treatment with oral antibiotic therapy is possible if there is no sign of severity or co-morbidity (diabetes, arteritis, cirrhosis, immune deficiency) or an unfavorable social context. In case of allergy to penicillin, pristinamycin or clindamycin should be prescribed. Prophylactic antibiotic therapy with delayed penicillin is recommended in the event of recurrent erysipelas.
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Scurvy is a clinical syndrome resulting from ascorbic acid deficiency. Although presently rare in the developed world, it continues to occur within certain susceptible populations, particularly the elderly and those with dietary habits with low vitamin C intake. ⋯ Diagnosis of scurvy can be challenging. This report highlights the importance of maintaining a high index of suspicion for scurvy in atypical necrotic ulcerations of lower extremities with poor response to standard therapy especially in populations at high risk of vitamin C deficiency. Early diagnosis can improve patient prognosis.